beta-N-Acetylhexosaminidases

A hexosaminidase specific for non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. It acts on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES. Two specific mammalian isoenzymes of beta-N-acetylhexoaminidase are referred to as HEXOSAMINIDASE A and HEXOSAMINIDASE B. Deficiency of the type A isoenzyme causes TAY-SACHS DISEASE, while deficiency of both A and B isozymes causes SANDHOFF DISEASE. The enzyme has also been used as a tumor marker to distinguish between malignant and benign disease.

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Networked: 414 relevant articles (2 outcomes, 25 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Zwierz, Krzysztof: 9 articles (10/2009 - 01/2006)
2.	Tsuji, Daisuke: 7 articles (08/2010 - 03/2005)
3.	Itoh, Kohji: 7 articles (08/2010 - 03/2005)
4.	Sakuraba, Hitoshi: 6 articles (08/2010 - 01/2002)
5.	Borzym-Kluczyk, Malgorzata: 5 articles (10/2009 - 01/2007)
6.	Seyfried, Thomas N: 4 articles (06/2010 - 01/2007)
7.	Baek, Rena C: 4 articles (06/2010 - 01/2007)
8.	Sandhoff, Konrad: 4 articles (11/2009 - 04/2003)
9.	Olszewska, Ewa: 4 articles (07/2009 - 01/2007)
10.	Zwierz, K: 4 articles (01/2007 - 09/2003)

Related Diseases

1.	Tay-Sachs Disease 05/16/1977 - "Tay-Sachs disease: an improved, fully-automated method for heterozygote identification by tear beta-hexosaminidase assay." 08/01/2008 - "To determine the structural changes in the alpha-subunit of beta-hexosaminidase due to amino acid substitutions causing Tay-Sachs disease, we built structural models of mutant alpha-subunits resulting from 33 missense mutations (24 infantile and 9 late-onset), and analyzed the influence of each amino acid replacement on the structure by calculating the number of atoms affected and determining the solvent-accessible surface area of the corresponding amino acid residue in the wild-type alpha-subunit. " 10/01/2005 - "The relative proportion in percentage of the isoenzyme A of beta-hexosaminidase (Hex) is the single discriminatory function most frequently used for the biochemical screening of heterozygote Tay-Sachs disease carriers. " 04/11/2003 - "Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease." 04/01/2003 - "Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease."
2.	Neoplasm Metastasis (Metastasis) 10/01/1986 - "Previous studies have demonstrated a variant form of beta-hexosaminidase in metastatic tumor tissue of human liver and in sera from cancer patients with liver metastases. " 10/01/1986 - "Examination of subgroups of the cancer patients based on the extent of metastasis revealed that there was a significant increase in total serum beta-hexosaminidase activity and the presence of variant beta-hexosaminidase in the sera as the disease progressed. " 11/01/1984 - "The variant beta-hexosaminidase may prove to be a useful general marker for tumor burden or a more specific marker for liver metastases." 11/01/1984 - "The present investigation was concerned with determining if this atypical beta-hexosaminidase could be detected (by analytical column isoelectric focusing) in the sera of cancer patients who have liver metastases. " 11/01/1984 - "Atypical beta-hexosaminidase in sera of cancer patients with liver metastases."
3.	Hepatitis 01/01/1981 - "The elevated beta-hexosaminidase levels are attributed to defective non-parenchymal liver cell function in hepatitis." 01/01/1981 - "The lysosomal enzyme beta-hexosaminidase was elevated in different forms of hepatitis. " 01/01/1981 - "beta-Hexosaminidase level in serum from patients with viral hepatitis as a measure of reticuloendothelial function." 09/01/1983 - "(3) The presence exclusively of precursors of beta-hexosaminidase and of cathepsin D in the sera of patients with hepatitis suggests that in hepatitis secretion of lysosomal enzymes is elevated, rather than the enzymes leaking from damaged cells. " 09/01/1985 - "Fifty-one patients (16 with malignant extrahepatic biliary obstruction, ten with benign extrahepatic biliary obstruction, eight with alcoholic liver disease, five with viral hepatitis and 12 with liver metastases) and 19 adult healthy controls were studied with determinations of beta-N-acetyl hexosaminidase (a lysosomal enzyme which is cleared from the circulation by the Kupffer cells), carcinoembryonic antigen (CEA), serum bilirubin, alkaline-phosphatase and aspartate aminotransferase (AST). "
4.	Alcoholic Liver Diseases (Alcoholic Liver Disease) 01/31/1984 - "The combined determination of serum beta-hexosaminidase and total serum bile acids is proposed for evaluating alcoholic liver disease." 01/31/1984 - "Total fasting serum bile acids and beta-hexosaminidase in alcoholic liver disease." 09/01/1985 - "Fifty-one patients (16 with malignant extrahepatic biliary obstruction, ten with benign extrahepatic biliary obstruction, eight with alcoholic liver disease, five with viral hepatitis and 12 with liver metastases) and 19 adult healthy controls were studied with determinations of beta-N-acetyl hexosaminidase (a lysosomal enzyme which is cleared from the circulation by the Kupffer cells), carcinoembryonic antigen (CEA), serum bilirubin, alkaline-phosphatase and aspartate aminotransferase (AST). "
5.	Neoplasms (Cancer) 11/01/1984 - "Previous studies have reported altered isozyme compositions and properties of beta-hexosaminidase in human cancerous tissues, and an atypical beta-hexosaminidase was found previously in metastatic tumor tissue of human liver. " 10/01/1986 - "Analytical isoelectric focusing indicated that the variant beta-hexosaminidase was present in 80% of 108 cancer patients, 37% of 27 pathological controls and 11% of 18 normal controls. " 10/01/1986 - "The current investigation examined sera for the presence of the variant form of beta-hexosaminidase from a large and heterogeneous group of cancer patients (with different primary sites and differing degrees of metastatic involvement), from normal controls and pathological controls with nonmalignant diseases. " 09/01/1985 - "These findings indicated that the formation of mixed disulfide bonds in the tumor beta-hexosaminidase B increased the net negative charge and resulted in the appearance of a heat-stable form." 09/01/1985 - "With respect to heat stability, beta-hexosaminidase B from normal lung was very stable at 52 degrees, while tumor beta-hexosaminidase B was unstable. "

Related Drugs and Biologics

1.	Alkaline Phosphatase
2.	Carcinoembryonic Antigen
3.	Bilirubin
4.	Aspartate Aminotransferases (Aspartate Transaminase)
5.	Isoenzymes
6.	alpha-Mannosidase (LAMAN)
7.	Hexosaminidase A (Hex A)
8.	Glucuronidase
9.	Antigens
10.	Serotonin (5 Hydroxytryptamine)

Related Therapies and Procedures

1.	Caloric Restriction
2.	Enzyme Therapy
3.	Transplants (Transplant)
4.	Laparoscopic Cholecystectomy
5.	Bone Marrow Transplantation (Transplantation, Bone Marrow)